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Question :

Posted On : 29 Sep 2005

In continuation with the last query:

Regarding the previous case I asked for, we did liver biopsy in her previous admission which was normal and we also repeated bone marrow biopsy that showed erythroid hyperplasia. Also smear for LD bodies were negative. Brucella work up was negative. Her sarcoidosis work up was also normal. Her complement levels are normal. We are waiting for c-anca and p-anca reports. And once at the age of 9 yrs she was treated as JRA due to knee joint swelling. 1 episode of GTCS plus at age of 9 yrs. I had enquired about macrophage activation syndrome also in view of this case. Thanks for ur reply.Now her urine protein also is elevated. So can we put her as JRA in the past now complicated with MAS ? Hope it`1l present with repeated infections. Now she is on oral steroids after iv Methyl Prednisolone and she is responding well we hope. KINDLY tell how else to confirm. Our bone marrow shows only erythroid hyperplasia...no evidence of hemophagocytosis.

Expert Answer :

No expert answer available.

Answer Discussion :

Dr Sanwar Agrawal

When I asked about the pre and post platelet count I had the possibility of MAS in mind bone marrow examination provides the confirmation. I am not convinced though that a monoarticular involvement should be labeleld as JRA

19 years ago

pediatriconcall

Check her serum ferritin levels to confirm MAS. However since she is already on methypred the levels may not be raised. However with hepatosplenomegaly, anemia, arthritis, CNS involment and renal involvement, autoimmune disease along with MAS is likely. Hemophagocytosis may not be always picked up on MAS if done early in the disease or the child is already on treatment.

19 years ago

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